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Epithelioid angiosarcoma of the cervical spine: A case report
Yu-Han Nan1, Cheng-Di Chiu1,2,3, Wei-Liang Chen4
1Department of Neurosurgery, China Medical University Hospital, Taichung 404327, Taiwan.
Background:
Epithelioid angiosarcoma (EA) is an aggressive, malignant endothelial-cell tumor of vascular or lymphatic origin. EA often arises from deep soft tissues such as pleura, breast, bone and gastrointestinal tract. It usually affects patients aged 60-70 years and is associated with high recurrence and metastasis rates with surgical resection as the primary treatment of choice. Overall survivals are generally poor, ranging from 6 to 16 months. More than 50% of patients died of disease within 2 to 3 years of diagnosis.
Case Summary:
We present a rare case of EA of the cervical spine causing a C6 pathological fracture complicated by severe kyphosis. The patient received C4-7 posterior laminectomy and C2/3/4/7/T1 transpedicular screw fixation, followed by anterior C5-6 corpectomy with allograft bone fusion and cervical plate fixation. Postoperative radiotherapy was administered without delay. However, the patient died of rapidly progressive acute respiratory distress syndrome 3 weeks after the second surgery.
Conclusion:
EA with spinal involvement is extremely rare. Early detection and cord decompression may prevent neurological deterioration and preserve better quality of life.
